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EB61.0
1.2.3 Localized scleroderma
Grading & Level of Importance: C
ICD-11
Synonyms
Morphea, scleroderma circumscriptum.
Epidemiology
Where present, most frequently between 20-40 years, 15%
Definition
Uncommon localized connective tissue disease with inflammatory border (lilac ring).
Aetiology & Pathogenesis
Unknown. In some cases tick bite induction proven.
Signs & Symptoms
Centrifugally spreading erythema with progressive central white-yellow induration with loss of adnexal structures. Border often with purple tones (lilac ring) as sign of disease activity.
Severe course with involvement of deeper structures possible, especially with linear scleroderma ("en coup de sabre" on scalp), facial hemiatrophy.
Localisation
Varies with classification.
Classification
1. Limited forms: plaque-type morphea, guttate morphea, atrophoderma of Pierini-Pasini
2.Generalized forms: generalized circumscribed scleroderma, disabling pansclerotic morphea, eosinophilic fasciitis
3. Linear forms: linear circumscribed scleroderma including type "en coup de sabre", Parry-Romberg syndrome
4. Deep morphea (see www.AWMF guidelines).
Laboratory & other workups
1. General
- routine laboratory tests
- Borrelia serology
- ANA
2. In children with severe linear forms: rheumatoid factor.
Dermatopathology
Interface dermatitis in early phases with varying intensity of inflammatory cellular infiltrates and changes of collagen structure depending on time of the disease course. Identical to progressive systemic sclerosis (see there).
Course
Plaque-type morphea: usually self-limited within years leaving sclerotic, pigmented scars. Linear circumscribed scleroderma and hemifacial atrophy: chronic with significant impairment of growth and function.
Complications
Depends on localization (face, overlying joints), depth and extension (pansclerotic morphea).
Generally, no systemic involvement (in contrast to progressive systemic sclerosis). In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, possible underlying bone and CNS involvement.
Diagnosis
Clinical features, histology. In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, cranial magnetic resonance imaging (MRI) and high resolution CT scan should be performed to document bone involvement and exclude CNS involvement.
Differential diagnosis
Lichen sclerosus (et atrophicus), acrodermatitis chronica atrophicans (borreliosis), dermatosclerosis associated to severe chronic venous insufficiency , progressive systemic sclerosis, eosinophilic fasciitis.
Prevention & Therapy
No causal therapy available.
- Topical therapy: corticosteroids; calcipotriol
- Intralesional corticosteroids
- Systemic therapy: methotrexate; corticosteroids; hydroxychloroquine
- Phototherapy: PUVA; bath PUVA; UVA-1
- Physical therapy: range of motion exercises; manual lymphatic drainage
Special
None.
Differential Diagnosis
Podcasts
Tests
- This young woman has morphea on her chest. What is the preferred treatment?
- Statement 1 Panmorphea is generalized morphea involving the entire integument
- 35-year-old woman presents with multiple brown plaques on back with sclerotic consistency. They have been present for months. Which of these examinations is indicated?
- Which lesions are seen in morphea?
- Statement 1 Morphea in the face does not require treatment
- 30-year-old patient with indurated plaque in lumbosacral region with slow centrifugal spread. The biopsy shows dermal sclerosis with loss of hair follicles. What is your diagnosis?
- True or false?
- This 16-year-old boy has noticed this lesion on his forehead for a few months. He denies any trauma. Your diagnosis?
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